Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

BACKGROUND: Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. METHODS AND RESULTS: We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤ 3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31 ± 10 months). Echocardiography performed 21 ± 13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. CONCLUSIONS: Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤ 2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤ 2 would benefit from prophylactic MV intervention.

Spontaneous rectus sheath hematomas: when to restart anticoagulation?

BACKGROUND: The aim of the present study was to obtain data regarding the timing of anticoagulation resumption in patients with spontaneous rectus sheath hematomas (RSH). PATIENTS AND METHODS: The study is based on review of patients receiving anticoagulation medication who were diagnosed with a spontaneous RSH (traumatic and iatrogenic excluded) between 14 July 1997 and 17 March 2012. RESULTS: There were 156 patients (37 % male; aged 73 ± 13 years) with an average body mass index of 29 ± 7 (procedure group 28 ± 6). Anticoagulants included coumadin (64 %), intravenous heparin (21 %), aspirin (8 %), and others (7 %). An intervention was needed in 29 (19 %) of the patients (5 % operative; 16 % embolization). Sixty-two percent of patients had their anticoagulation restarted during their hospitalization, with a median re-initiation time of 4 days after RSH diagnosis (range 2-8 days). Timing of anticoagulant resumption did not differ regardless of the need for intervention (3 vs. 4 days). The complication rate was 19 % (42 % in the procedure group, none specific to the procedure), with the most common being acute renal failure (n = 8; 5 %), death (n = 8; 5 %), and thrombotic events (n = 5; 3 %). After resumption of anticoagulation, two patients suffered enlargement of their RSH, both 2 days after resumption. CONCLUSIONS: Intervention to control hemorrhage was unnecessary in the majority of patients with RSH. In those with resumption of anticoagulation, the majority of patients were safely restarted by day 4. Even though complications secondary to anticoagulation were few, thrombotic complications outnumbered bleeding complications, suggesting that anticoagulation was withheld for too long after RSH diagnosis.

Nonoperative management of traumatic suprahepatic inferior vena cava pseudoaneurysms.

Inferior vena cava (IVC) pseudoaneurysms are rare clinical entities with an uncertain natural history due to limited follow-up information. This case report describes a 30-year-old woman with two IVC pseudoaneurysms and their associated anatomy. It also details our treatment plan, with follow-up through radiographic resolution of the pseudoaneurysms. To our knowledge, this is the first case report to document a patient with two pseudoaneurysms of the IVC and full resolution treated by nonoperative management.

Outcome of repair of myocardial bridging at the time of septal myectomy.

BACKGROUND: Myocardial bridging describes systolic compression of the muscular investment of a portion of an epicardial coronary artery. We evaluated the outcome of muscular bridge unroofing of the left anterior descending artery at the time of septal myectomy in patients with hypertrophic cardiomyopathy. METHODS: We conducted a case-controlled study of 36 patients (23 men; median age, 42 years) with hypertrophic cardiomyopathy and myocardial bridging. Group 1 patients had septal myectomy and concomitant unroofing (n = 13), group 2 patients underwent myectomy alone (n = 10), and group 3 patients were treated medically (n = 13). RESULTS: Angina was more prevalent preoperatively in group 1, 46% compared with 20% in group 2. Preoperative left ventricular outflow tract gradients of 67.8 ± 58.2 mm Hg and 74.1 ± 19.7 mm Hg were reduced to 1.9 ± 2.9 mm Hg in group 1 (p < 0.0001) and to 5.6 ± 8.8 mm Hg in group 2 (p < 0.0001). In the surgical groups, there were no early deaths or complications related to unroofing. Survival at 10 years was 83.3% in group 1 (p = 0.297), 100.0% in group 2, and 67.9% in group 3; there were no late sudden deaths. At follow-up, 77% in group 1 were asymptomatic compared with 70% of patients in group 2 (p = 0.19). There was no recurrent angina in group 1. CONCLUSIONS: Myocardial unroofing can be performed safely at the time of septal myectomy for left ventricular outflow tract obstruction. Angina was improved, but we found no difference in late survival compared with patients who had myocardial bridging and myectomy alone. Unroofing should be considered in patients with angina who have significant left anterior descending artery bridging and require myectomy.

Outcome of septal myectomy in patients with Fabry's disease.

Fabry's disease has cardiac manifestations that may mimic hypertrophic cardiomyopathy, including left ventricular outflow tract (LVOT) obstruction. It is important to distinguish hypertrophic cardiomyopathy from Fabry's disease, which can be treated with enzyme replacement therapy (ERT). However, after adequate ERT, some patients have no resolution of cardiac symptoms; thus, septal myectomy might be considered. We present 2 cases of extended septal myectomy in patients with LVOT obstruction secondary to Fabry's disease.

Transapical approach to myectomy for midventricular obstruction in hypertrophic cardiomyopathy.

BACKGROUND: Midventricular obstruction in hypertrophic cardiomyopathy (HCM) is less common than subaortic obstruction, and there are few data on outcomes after surgical treatment. METHODS: We reviewed 56 consecutive patients (28 men) with HCM and midventricular obstruction who underwent myectomy between February 1997 and June 2012. Five patients had prior myectomy for subaortic obstruction. Mean age was 42 ± 17 years. Preoperatively, 51% of patients had dyspnea, and the remaining had palpitations (25%), angina (5%), or syncope (9%). RESULTS: Midventricular obstruction was relieved by means of a transaortic myectomy in 5 patients, a transapical approach in 32 patients, and combined transaortic and transapical incisions in 19 patients. In 13 patients, an apical aneurysm or pouch was repaired at the time of midventricular myectomy. There were no early deaths. Intraoperative intraventricular gradients were reduced from 64 ± 32 mm Hg before myectomy to 6 ± 12 mm Hg postoperatively (p ≤ 0.0001). Early complications included atrial arrhythmias in 5 patients and reoperation for bleeding in 4 patients. Fifty patients had follow-up beyond 30 days (median, 1.6 years; range, 33 days to 13 years). Survival at 1 and 5 years was 100% and 95%, and average New York Heart Association class improved from 2.9 ± 0.7 preoperatively to 1.3 ± 0.6 postoperatively (p = 0.0001). There were no aneurysms related to the apical incision; 2 patients had late reoperation, 1 for resection of right atrial mass to prevent embolus. CONCLUSIONS: A transapical approach allows excellent exposure for midventricular myectomy and relief of intraventricular gradients and related symptoms. There were no complications unique to the apical incision, and 5-year survival was similar to expected survival (95% versus 97%).

Sleeve lobectomy: current indications and future directions.

Sleeve lobectomy was initially conceived as an alternative to pneumonectomy for patients with low-grade, centrally located lesions and limited cardiopulmonary reserve. Over the last several decades, advances in patient selection criteria and surgical techniques have allowed sleeve lobectomy to evolve from a compromise to pneumonectomy to first line intervention for centrally located lesions of all grades. Although more challenging than pneumonectomy, long-term outcomes and cost-effective measures favor sleeve lobectomy. The use of sleeve lobectomy has been expanded for locally advanced disease, and results remain superior to alternative procedures. Current literature has also shown evidence supporting the use of neoadjuvant treatment and minimally invasive techniques. It is likely that future results will continue to improve making sleeve lobectomy an even more attractive treatment option for qualifying patients.